Scleroderma is a rare, chronic autoimmune disease that affects skin and internal organs. Scleroderma results from the immune system causing inflammation and tissues changes. It often leads to skin tightening and thickening, and sometimes can affect joints, muscles, heart, lungs, kidneys, blood vessels or intestines. It affects mostly women between the ages of 30 and 50. While there is no cure for scleroderma, there are treatments to help manage symptoms and improve quality of life.
Most people with scleroderma can generally be classified based on the extent of involvement and the accompanying pattern of organ involvement. The major subsets include limited cutaneous SSc, Diffuse cutaneous SSc, SSc sine scleroderma, SSc overlap syndrome.
Signs/Symptoms?
Scleroderma symptoms may range from minor to life-threatening. Skin involvement is nearly a universal feature of SSc. The finger, hands, mouth are generally the earliest areas of the body involved. An early, common sign of scleroderma is color changes to the fingers called Raynaud’s phenomenon. Fingers or toes may look red, white, or blue, especially in cold temperatures and may be swollen, numb, painful or develop ulcers. In addition to Raynaud’s phenomenon, patients can have other symptoms depending on the involvement of joints, muscles, and internal organs including the heart, lungs, blood vessels, gastrointestinal tract, and kidneys. Nearly 90 percent of patients with SSc have evidence of GI involvement. Evidence for Pulmonary involvement is observed in 70 percent of patients with SSc. Diagnosis is based on history and physical exam findings, blood tests, autoantibodies seen in scleroderma, and imaging of heart or lungs.
Treatments
For Raynaud’s, blood pressure medications called calcium channel blockers or PDE-5 inhibitors like sildenafil (Viagra) and tadalafil (Cialis), can improve circulation. Antacids and proton pump inhibitors (omeprazole) can help with heartburn. There are no approved medications for skin thickening, but methotrexate and mycophenolate mofetil can be used early in the disease depending on the presence of other symptoms such as joint, muscle, or lung disease. Mycophenolate is also used to treat patients who develop scarring or inflammation of the lungs (known as interstitial lung disease). Other treatments for a serious lung complication (pulmonary arterial hypertension) can relax constricted blood vessels to ease high blood pressure.
Patients with Raynaud’s phenomenon should keep their bodies, hands, and feet warm with layered clothing, boots, and gloves in cold weather. Protect fingers and toes from activities that could injure skin. Keep skin moisturized. People with digestive problems might change their diet to prevent heartburn. Eating several smaller meals instead of three large ones, as well as not eating right before bed, can also help with heartburn. Regular exercise and physical therapy may keep joints flexible. Additionally, depression or mood problems are possible. Seek support from family or friends, patient groups, or if needed, mental health treatment from a psychologist. It is important to remember that it is possible for patients with scleroderma to lead long, fulfilling lives.